ESPE2022 Poster Category 2 Adrenals and HPA Axis (27 abstracts)
A Rare Cause of Peripheral Precocious Puberty
Seçil Çakır Gündoğan 1 , Aylin Kılınç Uğurlu 1 , Ayşe Özdemir Gökçe 2 , Derya Özyörük 3 , Gönül Yardımcı 1 , Yusuf Kasap 4 , Esra Karakuş 5 , Neriman Sarı 3 , Ayşe Seçil Ekşioğlu 2 , Erkan Ölçücüoğlu 4 & Mehmet Boyraz 1
1Ankara City Hospital, Pediatric Endocrinology Clinic, Ankara, Turkey; 2Ankara Yıldırım Beyazıt University, Pediatric Radiology Clinic, Ankara, Turkey; 3Ankara City Hospital, Pediatric Hematology-Oncology Clinic, Ankara, Turkey; 4Ankara City Hospital, Urology and Renal Transplantation Clinic, Ankara, Turkey; 5Ankara City Hospital, Patology Clinic, Ankara, Turkey
Introduction: Peripheral precocious puberty is a non-gonadotropin-dependent early pubertal development. Exogenous intake of sex steroids, gonadal or adrenal pathologies, ectopic hCG-secreting lesions play a role in the etiology. Adrenal tumors is a rare cause of heterosexual precocious puberty.
Case: An eight-year-old female patient was admitted with the complaints of genital hair growth and breast enlargement. The patient's history and family history were unremarkable. On physical examination, body weight was 34.5 kg (+1.28 sds), height was 137.2 cm (+1.41 sds), puberty Tanner stage3, hyperpigmentation in the areola and cliteromegaly were detected. In the laboratory tests of the patient, FSH 0.8 U/l, LH < 0.07 U/l, Estradiol 470 ng/l, Total Testosterone 389 ng/ml, DHEA-S 262.02 µg/dl, Androstenedione 30.2 nmol/l, DHEA 6.60 mg /l, 11 deoxycortisol 4.32 ng/ml, 17-OH progesterone 6.29 nmol/l, ACTH <5.00 pg/ml Cortisol 18, 5 µg/dl,24-hour urinary cortisol 539 mg /m2 detected. Result of the one mg dexamethasone suppression test is ACTH<5.00 pg/ml Cortisol 16.2. Bone age is 12 years old, and in the pelvic ultrasonography the uterus 35x24x26mm, endometrium 5 mm, right ovary 5.7 mm, left ovary 3.3 ml was found. At the same time in the abdominal ultrasonography, a heterogeneous hypoechoic mass with 43x34 mm calcifications was observed in the right adrenal. With advanced imaging methods, it was seen that the mass was localized in the adrenal gland. The patient underwent mass excision. In the laboratory examinations of the patient one week after the operation, Estradiol 11.0 ng/l, Total Testosterone <0.07 mg /l, DHEA-S 6.74 µg/dl, Androstenedione <1.05 nmol/l, DHEA 0.88 mg / l, DHEA-S 6.74 µg/dl, 11 deoxycortisol 0.0943 ng/ml. The pathology result was compatible with adrenocortical carcinoma and genetic analysis was sent from the patient in terms of p53 mutation. The patient is under follow-up by our endocrinology and oncology department.
Conclusion: Adrenocortical tumors are a very rare cause of childhood malignant tumors. Weiss criteria are the most preferred method in the differentiation of malignant/benign tumor. The clinical course of a case with extremely rare seen malignant adrenocortical carcinoma in the etiology of peripheral precocious puberty, is shared.
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